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25 Cards in this Set
- Front
- Back
- 3rd side (hint)
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T cell testing for immunodefic |
Quant- flow cytometry Qual- delayed type hypersensitivity (candida, mumps) Tertiary tests - specific enz measurements, mitogens |
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B cell testing for immunodefic |
Quant- quant immunoglobulins Qual- response to vaccines Tertiary- neoantigen studies, mitogens |
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Phagocyte testing for immunodefic |
Quant- cbcwdiff Qual- neutrophil oxidation (nbt, dhr) Tertiary tests- surface glycoproteins |
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Complement testing for immunodefic |
Quant- c3, c4 Qual- ch50 tertiary- individual compl |
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Severe combined immunodeficiency (scid) |
X-linked, AR, Ada defic, pnp defic Sepsis in infancy, eczema, chronic lung infxn, absent thymus on CXR, lymphopenia |
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Omenn syndrome |
variant of RAG deficiency Erythroderma, FTT, diarrhea, HSM Hypo gamma globulin emus, hi IgE, hi eos |
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Wiskott-Aldrich syndrome |
E- eczema X- x-linked I- immunodeficiency T- thrombocytopenia |
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Ataxia-telangectasia |
AR, in ATM gene |
Ataxia+telangectasia+hi alpha fetoprotein by age 5 Sinopulm dz % bronchiectasis common Some immunodeficiency Hi risk leukemia/lymphoma |
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Bloom syndrome |
DNA ligase defic Small stature, telangectasias, CNS anomalies, immunodeficiency Hi risk leukemia |
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Nimegen breakage syndrome |
AR, defective dsDNA repair Bird like facies, microcephaly, normal IQ Hi risk malignancy |
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Briton's agammaglobulinemia |
X linked, BTK tyrosine kinase defect P/w encapsulated bac infxns (AOM, PNA) or recurrent giardia & enterovirus Rx w IVIG |
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Common variable immunodeficiency |
Recurrent sinopulm infxn B cell problem Defic in at least 2 Ig classes & poor IG fct (lo vaccine titers) Rx w IVIG |
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Hyper IgM syndrome |
AR, x linked -> B cell problem Defic IgA & IgG, nl IgM HSM, LAD, recurrent sinopulm infxn, giardia, PCP, chronic parvo Rx IVIG, bactrim |
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X linked lymphoproliferative d/o (Duncan syndrome) |
X linked Severe/fatal EBV w fulm hepatitis, b cell lymphoma, agranulocytosis, a plastic anemia Burkitt lymphoma rism |
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Leukocyte adhesion defect |
Chemotaxis defect Delayed umbilical cord separation Baseline hi WBC Recurrent necrotizing inxns of skin, mucosa, gut, lung w/ cigarette paper scarring |
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Hyper IgE syndrome (job syndrome) |
STAT3 defic -> killing defect Eczema, delayed dental exfoliation, pneumatocele, dysmorphic Cold abscess w staph, hib, s pneumo |
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Chediak higashi syndrome |
Killing defect w large neutrophil granules on smear Recurrent skin & sinopulm infxn Periph neuropathy Ptl oculocutaneous albinism |
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Chronic granulomatous dz |
X linked Superoxide defect Chronic organ & skin abscess w staph, serratia, burkholderia, aspergillus DX NBT or DHR Rx BMT |
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Hereditary angioedema defect |
AD Defect in c1 inhibitor fct > lo c4 |
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Hereditary angioedema types |
1- decreased c1 inhibitor enz 2- non functioning c1 enz |
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Hereditary angioedema rx |
Prevention: Danazol (attenuated androgen) incrs c1 inhibitor levels Acute: purified c1 inhibitor enz |
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Type I hypersensitivity |
IgE mediated Immediate (anaphylaxis, a topic) |
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Type II hypersensitivity |
IgG mediated Cytotoxic |
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Type III hypersensitivity |
Immune complex (Ab-Ag) mediated |
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Type IV hypersensitivity |
Cell mediated |
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